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The deep brain stimulation (DBS) in movement disorders is well established and in many aspects evidence-based procedure. The treatment indications are very heterogeneous and very specific in their course and therapy. The deep brain stimulation plays very important, but usually not the central role in this conditions. The success in the application of DBS is essentially associated with the correct, appropriate and timely indication of the therapy in the course of these diseases. Thanks to the good standardization of the DBS procedure and sufficient published data, the recommendations for indication, diagnosis and operative procedures can be generated. The following article attempts to summarize the most important decision-making criteria and current therapy standards in this fairly comprehensive subject and to present them in close proximity to practice., Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt vorliegt., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Animal experiments have shown that after specific nerve traumatization, neuromuscular electrostimulation (NMES) can promote nerve regeneration and reduce synkinesia without negatively interfering with normal regeneration processes. NMES is used routinely in physical rehabilitation medicine., Methods: This systematic literature search in the Cochrane Central Register of Controlled Trials, the Cochrane Database of Systematic Reviews, the DAHTA database, the Health Technology Assessment Database and MEDLINE or PubMed considered studies on the use of NMES in otorhinolaryngology that have been published in German or English., Results: The search identified 180 studies. These were evaluated and relevant studies were included in the further evaluation., Discussion: In the fields of otorhinolaryngology and phoniatry/paediatric audiology, clinical studies investigating the effects of NMES on facial and laryngeal paresis, as well as dysphonia and dysphagia have been carried out. The evidence collected to date is encouraging; particularly for the treatment of certain forms of dysphagia and laryngeal paresis.

A number of genetic causes of movement disorders including Parkinson disease, dystonia, restless legs syndrome or essential tremor have been elucidated in recent years. This process was accelerated by novel technologies including genome-wide association studies (GWAS) and next generation sequencing (NGS). Although monogenic forms are overall rare, they provide a unique opportunity to investigate mutation carriers who are still in the presymptomatic phase. As these subjects present individuals at risk to develop the disease, they have been included in longitudinal studies to unravel disease mechanisms and elucidate novel therapeutic targets. In addition, cell culture and animal studies have been performed to functionally characterize proteins mutated in different movement disorders to provide further insight into disturbed cellular pathways. In this article, we summarize known monogenic forms and the associated phenotype as well as genetic risk factors and review the function of relevant genes and proteins.

Deep brain stimulation (DBS) is a safe and successful therapeutic option for patients with dystonia and tremor syndrome who do not respond sufficiently to conservative therapies. The most common target of DBS in patients with dystonia is the internal region of the globus pallidus (GPI). DBS of the GPI leads to long-lasting and remarkable improvement of dystonic movements in about 80% of patients. Recently it could be shown that not only patients with idiopathic dystonia but also patients with secondary dystonia can benefit from DBS although to a somewhat lesser extent. In patients with tremor syndromes, such as essential tremor, tremor-dominant Parkinson's disease or tremor in multiple sclerosis (MS) the intermediate ventral nucleus of the thalamus (VIM) as well as the subthalamic region proved to be promising targets for DBS electrodes. Especially in patients with essential tremor VIM-DBS leads to an often acute reduction of the tremor syndrome. In long-term observations, however, patients with essential tremor showed some tolerability to VIM-DBS leading to a slow increase of stimulation parameters to maintain a stable effect. VIM-DBS in patients with Parkinson's disease is rare and is reserved for elderly patients with pronounced tremor syndrome and little disease progression. Controlled studies and data on DBS in MS tremor are lacking and data are sparse and heterogeneous. Therefore, VIM-DBS in MS tremor patients has to be evaluated individually with caution. In summary patients with tremor syndromes as well as dystonia who cannot be adequately controlled with conservative therapy are good candidates for deep brain stimulation, a therapeutic option with moderate complications and risks and very good outcome for most patients.

In patients with primary generalized or segmental dystonia controlled studies have shown that bilateral pallidal deep brain stimulation (DBS) can reduce disability scores by 40 to 60 %. Up to now, the data base on the therapeutic effects of DBS in selected patients with focal dystonia is less extensive. Long-term efficacy of DBS in dystonia has been demonstrated, but reports have focused mainly on patients with primary generalized dystonia so far, with a mean observation period of approximately 5 years. Predictors of a favourable long-term efficacy of DBS in primary generalized dystonia patients are young age at surgery, a shorter duration of the disease, a lower severity of disability, and a posteroventrolateral localization of the DBS electrode within the globus pallidus internus (GPi). Controlled studies are needed to identify also the factors determining the best long-term outcome of non-generalized dystonia patients treated with DBS., ((c) Georg Thieme Verlag KG Stuttgart-New York.)

Medical treatment of dystonia, particularly generalised forms of the disorder, is often not satisfactory or causes intolerable side effects. In focal dystonia, a reasonable treatment option with botulinum toxin exists but some patients either do not respond well or develop neutralising antibodies with secondary therapy failure. Deep brain stimulation (DBS) of the globus pallidus internus has been shown to be effective in both generalised and focal dystonia. This paper gives recommendations regarding the use of DBS in different forms of dystonia based on the currently available scientific data as well as the longstanding personal experience of the authors. The inclusion criteria for DBS candidates as well as the peri- and postoperative patient management are addressed. These recommendations were developed in a consensus procedure in the German Deep Brain Stimulation Association.

In Germany, deep brain stimulation (DBS) of the thalamic ventralis intermedius nucleus (VIM) is licensed for treatment of essential tremor in cases unresponsive to pharmacotherapy. Especially a bothersome hand tremor interfering with activities of daily living will improve, whereas head, tongue or vocal tremor shows less response. DBS was proven to be superior to lesional thalamotomy with better functional outcome and less adverse effects. The consensus statement presented here reflects the current recommendations of the German Deep Brain Stimulation Study Group for inclusion and exclusion criteria as well as for peri-, intra- and postoperative neurological management.

Dystonia, a hyperkinetic movement disorder, is characterized by involuntary muscle spasms leading to abnormal postures. Dystonic syndromes are classified by etiology (primary vs. secondary), age of onset (early vs. late onset) or spread of symptoms (focal, segmental, generalized). Clinically, young-onset dystonia is rare, often inherited and tends to spread to become generalized. In contrast, adult-onset dystonia is frequent, typically sporadic and remains focal. In recent years, 15 genes associated with dystonia have been identified and classified as DYT loci. Of these, DYT1 is the most frequent, causing early-onset generalized dystonia. Pathophysiology remains ill understood but basal ganglia dysfunction is thought to play an important role. Treatment remains symptom-oriented. A trial of levodopa is recommended in young-onset cases. In focal forms, botulinum toxin injections are helpful. Anticholinergics may be beneficial. In severe cases deep brain stimulation may be considered.

Despite great advances in the understanding of the dystonias, treatment for most patients remains symptomatic. Botulinum neurotoxin (BoNT) is the treatment of choice for focal dystonias. Selected issues related to the use of BoNT are highlighted with pertinent illustrations. In certain focal, mainly in generalised dystonias BoNT may not be sufficient or appropriate. Besides BoNT pharmacotherapy, nowadays increasingly deep brain stimulation, psychotherapy, physio- and occupational therapy approaches can be included in the management of dystonic patients. The review focuses on experiences with established management guidelines and new developments in the treatment of dystonias.

Disturbance of posture may occur in a variety of neurological disorders and occasionally is the presenting or even the only sign. In the majority of cases, the head or the trunk or both are bent forward (bent spine syndrome, dropped head syndrome). A feature of these primary neurogenic or myogenic postural disturbances that is in contrast to antalgic contraction or ankylosis is that they are not fixed, but the trunk or head are easily erected by the examiner and show a characteristic sagging. Neuromuscular disorders are a frequent cause. They may be confined to the paraspinal muscles. Axial computed tomography of the spine, electromyography of the involved muscles, and muscle biopsy help to make the diagnosis. However, also central movement disorders may lead to a sagging of the head or trunk or of both due to a lessened tone of the head and trunk extensors. This is frequently seen in the various parkinsonian syndromes which may, however, occur in association with a focal myopathy of the paraspinal muscles. Occasionally, sagging of the trunk is seen as a side effect of neuropharmacologic medication. Sagging of the trunk or head should be differentiated from a pathologically increased innervation of the ventral muscles in dystonic movement disorders such as antecollis or camptocormia. Pathologic reclination of the head or trunk or both is a rare disturbance of posture. It may occur in dystonia (retrocollis) or, occasionally, as a consequence of musculotendinous contractures secondary to certain neuromuscular disorders such as the rigid spine syndrome.

Dystonic movements and other dyskinesias often cause diagnostic difficulties due to their complex symptomatology. Namely focal dystonias are frequently misdiagnosed as conversion reactions. Idiopathic dystonias are generally considered a nonstructural (neurobiochemical) disorder of the basal ganglia. Many case reports, however, have dealt with patients presenting with "atypical" dystonia whose symptoms were relieved by psychotherapy or hypnosis. We present the histories of two young women exhibiting focal dystonia emerging for the first time under circumstances of profound emotional trouble. We discuss the general difficulties in the diagnosis of dystonic movement disorders and review the criteria for the diagnosis of "psychogenic dystonia". The basal ganglia integrate limbic, proprioceptive and sensorimotor inputs to create emotionally and functionally appropriate voluntary movements. Therefore, the traditional dichotomy "psychogenic-somatogenic" appears to be inappropriate when applied to extrapyramidal movement disorders. In a psychosomatic understanding, the assumption of a psychogenic "trigger" for a somatic movement disorder does not mean a contradiction.

The focal dystonias of cranial-nerve innervated muscles are described. They include the ocular dystonia, blepharospasmus and other facial dystonias, mandibular dystonia, pharyngeal dystonia, spasmodic dysphonia, external laryngeal dystonia, spasmodic torticollis and lingual dystonia. They share similar clinical aspects such as the inadequate co-contraction of antagonistic muscles, dystonic overflow of muscle activity to muscles not normally involved, similar facilitating and inhibitory activities and various "antagonistic gestures". Genetic, imaging, neuroanatomic, physiologic and pharmacologic findings suggest common pathogenetic mechanisms for these diseases. Similar therapeutic approaches are established. The frequent and variable combinations of these focal dystonias and their similar pathogenetic background favour their common classification as craniocervial dystonias.

For more than 100 years there has been a discussion as to whether writer's cramp is caused by a disease of the central nervous system, or if it is to be considered as a disturbance of psychogenic origin. Whereas before 1982 there seemed to be a lot of evidence for the psychogenic theory many authors now tend to stress the opinion of Sheehy and Marsden who had explained that "writer's cramp" should be seen as a "focal dystonia". This article discusses the statements of Sheehy and Marsden in comparison with other scientific findings regarding the occupational cramps--above all "writer's cramp". It is concluded that there are different therapeutic approaches derived from the respective theory and which are considered to lessen the movement disorder. Scientific literature suggests that psychotherapy or some therapeutic approaches of behaviour therapy are more effectful in mastering this "mysterious" disorder than any pharmacological substance tested upto now.

Craniocervical dystonias are a major therapeutic problem, since in the majority of cases various drug regimens as well as surgical interventions either fail or are accompanied by serious side effects. Botulinum toxin, well known as a biological toxin which blocks the cholinergic neuromuscular synapse, proved to be a new and successful concept in the treatment of these disorders. In the present study, mechanisms of action, clinical indications and practical use of botulinum toxin are described.